The epidemiology of glomerulonephritis and rare renal disease at a large tertiary centre

Abstract

Glomerulonephritis refers to pathology of the glomerulus, the basic structural unit of the kidney, and is a term used to encompass several different disease entities. These were first classified according to light microscopy and immunofluorescence staining findings, but with increased understanding of the underlying pathophysiology in some cases there has been a move to classification based on biomarkers or antibodies. In this thesis, there is a focus on four specific types of glomerulonephritis: Immunoglobulin A nephropathy (IgAN), membranous nephropathy (MN), focal and segmental glomerulosclerosis (FSGS), and anti- neutrophil cytoplasmic antibody associated vasculitis (AAV). These conditions are all rare, making study of their epidemiology and natural history difficult. Furthermore, standardised management approaches have been introduced in the last decade or so, further distorting the previously published literature. This thesis aims to provide a comprehensive real-world update on the epidemiology, management and outcomes of these conditions. Comprehensive retrospective datasets over at least 20 years were established. Epidemiological studies were undertaken using patients diagnosed at Salford Royal Hospital with IgAN, MN, FSGS and AAV. The long study period had a number of benefits including allowing relatively large datasets to be developed, and to facilitate observation of the natural history of these conditions. These studies are presented in chapters 4.1-4.4. Chapter 4.5 focuses on IgAN by presenting the results of an analysis of healthcare associated costs for patients with this condition. The study contains the largest dataset of costs associated with IgAN when compared to the previously published literature, and is particularly relevant with the development of novel therapies for IgAN, which are likely to be higher cost than current supportive therapies. The final results chapter, 4.6, presents a real-world study of patients with Kidney Disease of Unknown Aetiology (KDUA)- a catch all term which is likely to include many undiagnosed rare kidney conditions. Demographics and outcomes are reported, and factors that may lead to improved diagnostic rates are investigated. Whilst there is still much to discover about glomerular and rare kidney diseases, this thesis demonstrates that a more granular understanding of the epidemiology and natural history of these conditions will complement the research being undertaken to further knowledge of the pathophysiology and novel drug targets, which when combined will ultimately lead to improved outcomes for patients.

Date of Award	1 Aug 2024
Original language	English
Awarding Institution	The University of Manchester
Supervisor	Philip Kalra (Supervisor) & Smeeta Sinha (Supervisor)